Sophie's Story, Aged 4


Diagnosis
It was a normal Friday in November 2011, Sophie had been at Preschool, we came home had lunch and then set off for her swimming lesson. There was absolutely no signs of anything going wrong. I got her changed and noticed there was a purple rash appearing on her legs, it had a somewhat raised appearance and while she wasn't complaining, it was painful, she didn't want me to touch it. We went straight to Accident and Emergency (10-15mins away); by the time she got out of the car, she was unable to walk. The Dr examined her, and told us she has Henoch Schoenlin Purpura. He gave us some written information on it and basically told us it could last up to 6 weeks, common symptoms associated (vasculitis, joint pain, abdominal pain...) and to treat with Paracetemol and Ibuprofen (we were later told not to treat with Ibuprofen as it induces bleeds with HSP children). The Dr skimmed over the rarer side effects which he assured us was unlikely to happen. We were sent home, and Sophie coped very well with it. The vascultiis spread from her feet to her bottom, and her arms, her hands and feet became very swollen. She had days of being completely immobile and days where she could walk with assistance. Things went on like this for about 2 weeks.

Abdominal Pain and Sickness
Sophie went off her food and began vomiting a few times a day. She was still able to tolerate fluids and minimal food. Things gradually got worse and we took her to the children's hospital every second day because we felt something just wasn't right. Every time we were sent away telling us that this was part of her condition and to basically treat it at home, there was nothing they could do for her. After four failed hospital visits, her condition deteriorated dramatically. She was very lethargic, fluid intolerant, could not keep her medicine down, she was very pale, almost grey. So she was finally admitted. The put her on IV fluids, gave her paracetemol rectally and anti sickness meds. Every venflon she had in tissued due to the poor state of her veins, she was still vomiting, she didn't sleep at all because she was so uncomfortable. At 4am, we were sent for an x-ray. At 11am we were sent for an abdominal ultrasound. The ultrasound tech looked at the screen and looked at my emaciated daughter, and I knew right away something was wrong.

Intussusseption and Surgery
As soon as we got back to ward, the Dr that continued to send us home insisting nothing was wrong, told us that she would be transferred to another hospital by ambulance for emergency surgery to correct her bowel. She has an intusussception, one of the rarer side effects the original Dr skimmed over with us. In the meantime she would be receiving emergency medication fluids, antibiotics and other things that I was just too upset to take in. Bags of fluids that usually last 4 hours were being pumped into my child in 20min. And still, she just lay there, no fight, no energy, vacant eyes. Within 30min of arriving at the other hospital, Sophie was under general anaesthetic and being operated on. We were told to get a coffee and wait in the ward, she would be back in around an hour. 5 hours passed, no answers, then the surgeon came in and shut the door and told us to sit down. Thinking of that moment still terrifies me now. He said our daughter had to have her bowel resectioned and reconstructed due to the intusussception turning necrotic because it was left untreated for so long. He told us that she had to be assisted in her breathing during surgery due to getting into difficulty. Her appendix had to be removed because the necrotic tissue adhered to it. He told us she was a very lucky little girl. We had come so close to losing her. She managed to wake well and thankfully avoided the High Dependency Unit. She came back to us with a nasogastric tube and bag, needle in her arm, needles in her feet, she was catheterized, on Morphine, and of course, a wound on her tummy. She was still vacant, there was still nothing there, but she had come back to us and that was main thing.

Discharge and Readmission
She was discharged the week of Christmas, and while still sore, she was picking up and began to become our little girl again, the one that sort of disappeared 3 weeks earlier. Christmas day was one of her better days, and for that I'm thankful. The sickness returned on New Years Eve, she was again lethargic. We went straight to the hospital where she was operated on. This time she had constipation and they were not willing to let us ride it out at home given her previous history. This led to another 10 day stay, during which time Drs discovered she had kidney involvement due to HSP. Her blood albumin was low, her Protein Creatinine Ratio was through the roof. She was moved to the Renal Ward to be started on steroids. 3 days nof IV Methyl Prednisolone followed by oral Prednisolone. She was also diagnosed with Hypertension linked to HSP and was started on Amlodipine for that, along with laxatives and Ranitidine to protect her tummy from the steroids. She picked up very quickly after the intense IV steroids, her purpura all but disappeared, sickness went away, and she started going to the toilet again.

HSP Nephritis and Nephrotic Syndrome
She was seen weekly by a Consultant Nephrologist, and also by a community nurse at home for Blood Pressure and urine checks. Her PCR levels continued to be up then down, then up again, and so on. Ranging from 600 and their lowest to 3400 at its highest. At her Thursday appointment, her Dr decided upon a kidney biopsy that would be scheduled for sometime in the next 2 weeks. The following Monday, we were about to leave for Nursery and the phone rang, I thought - just leave it, we are going out. I shut the door and my mobile rang, I answered and it was the Dr asking us to take her up that day for the biopsy. In a few hours, she was under general anaesthetic in theatre. That was an overnight stay and she was well after the procedure. At our following appointment HSP Nephritis and Nephrotic Syndrome Grade III was confirmed. A few weeks later her PCR, blood pressure and albumin were still unstable and she was started on another medicine called Enalapril which is primarily a Hypertension medicine but it has a secondary function to help stop proteins leaking from the injured kidneys. She has been on this almost 3 weeks now, and it has been upped 3 times so far. Her blood pressure is on the upper side of normal, but its stable. Her kidneys are still leaking a significant amount of blood and protein, but we have been told not to expect an overnight cure. Having been diagnosed in November, she will be on steroids till at least July. That takes us up to where we are right now (February, 2012).

Chicken Pox
Steroids are immunosuppressants and one of the conditions that children on steroids must be very careful of is Chicken Pox. My daughter had never had Chicken Pox and blood tests showed she had no immunity at all. Her nursery teacher called me on the weekend to tell me she had read on Facebook that one of the kids in nursery had her spots come out on the Friday night. I called the hospital who said she must be treated with an immunoglobulin injection to ensure she didn't get it. 3 weeks later and chicken pox is still ongoing with perhaps 5-6 kids contracting it, so she had to be given another injection and she will continue to get these injections until Chicken Pox is no longer in nursery.

Where we are today...
At this present moment we are still attending the hospital weekly for BP checks, urine PCR, weekly bloodwork. She is on 7 daily medications, some of which will continue to be increased and decreased with the possibility of adding some more into the mix depending on where her results take us. We haven't won the war on HSP yet, but we are putting up a hell of a fight and winning the little battles it continually throws at us. I have cried enough to last me a lifetime, my 4 year old hasn't shed a tear. She has coped brilliantly with it and has really set an example to adults around her about just getting up and getting on with it. It's really important to have active sites and active forums on HSP because I was clueless, I'd never heard of it and I didn't know what to do, who to speak to or the correct questions to ask. I don't want people to read our story, to be scared because it sounds like a horror story, it's not, its a success story.